Haematopathology Unit, Hospital Clinic . Barcelona University, Villarroel, 170, 08036 Barcelona, Spain +34 663 109 312 +34 932 275 717
Expert Rev Hematol. 2013 Dec;6(6):735-45. doi: 10.1586/17474086.2013.845522. Epub 2013 Oct 30.
Splenic marginal zone lymphoma (SMZL) is a lymphoma recognized as a distinct entity in the WHO classification of the lymphoid tumors. SMZL probably results from the expansion of a marginal zone B-cell driven by persistent antigen stimulation. SMZL is clinically and biologically heterogeneous. The SMZL Working Group has published guidelines for the diagnosis, workup and treatment of SMZL. There are no standard criteria to initiate treatment. A policy of watch and wait in asymptomatic patients is recommended. In symptomatic patients, data from retrospective studies suggest that rituximab with or without chemotherapy is the best strategy for SMZL. It is uncertain which is the optimal type of chemotherapy and whether patients may benefit from splenectomy prior chemoimmunotherapy. In the future, we may see progress with agents targeting known molecular lesions in SMZL.
脾边缘区淋巴瘤（SMZL）是一种在世界卫生组织淋巴肿瘤分类中被认定为独特实体的淋巴瘤。SMZL 可能是由持续抗原刺激驱动的边缘区 B 细胞扩增引起的。SMZL 在临床上和生物学上具有异质性。SMZL 工作组已经发布了 SMZL 的诊断、检查和治疗指南。目前尚无启动治疗的标准标准。建议对无症状患者进行观察和等待。对于有症状的患者，来自回顾性研究的数据表明，利妥昔单抗联合或不联合化疗是 SMZL 的最佳策略。尚不确定哪种化疗类型最佳，以及化疗前脾切除术是否对患者有益。在未来，我们可能会看到针对 SMZL 中已知分子病变的药物取得进展。
